Waging war against life-threatening lupus

Karen Birney of Narrowsburg is certain there are times people notice her in her motorized scooter — and are convinced there's nothing wrong with her.

Deborah J. Botti

Karen Birney of Narrowsburg is certain there are times people notice her in her motorized scooter — and are convinced there's nothing wrong with her.

Because if she's out and about, it's a good day living with lupus. She'll be wearing jeans, makeup, and chattering animatedly about whatever's going on.

"They don't realize what's going on inside," she'll often say to her close friend, Nancy Esposito of White Sulphur Springs, of the questioning looks she receives from strangers.

But in a matter of a day or two, what's going on inside can flare up and manifest on the outside, as well. Karen's face might suddenly be swollen, and one side a little droopy.

"There will be times when you are OK; and then you are not," Karen says. "You may not even be able to get out of bed."

This most recent flare-up has been particularly challenging, says Esposito of the past three months.

In September, Karen and her husband, Scott, were finally able to take an anniversary cruise.

"She had a few issues while away, but nothing major," says Esposito.

Karen also attended a birthday party in September — walking with a cane and almost glowing, says Esposito.

But a month later, she was barely able to attend the Walk With Us to Cure Lupus fundraiser in New York City. Friends who participated in the walk could not believe it was the same Karen.

Since then, she's been in and out of the hospital. Her leg pain is often excruciating — she's had a pain pump implanted — because of vasculitis. Vasculitis, or the inflammation of blood vessels, can also lead to stroke — which it did, about two years ago, for Karen.

"I just found out that the vasculitis is now in both my legs," says Karen. "This means I will need to go on chemo and Prednisone."

Adds Esposito, "There's also been a new diagnosis regarding her heart, so they're worried about starting chemo, which some lupus patients respond well to with these symptoms. Others don't. Given all the side effects, chemo is risky. "» Karen hates all the medications, but she wants to be there to see her 18-month-old granddaughter grow up."

Lupus is defined as a systemic autoimmune disease. The immune system encompasses the arsenal that equips the body to defend itself against unwanted invaders, such as viruses, bacteria and parasites. Sometimes the immune system goes haywire and starts attacking itself.

Many autoimmune diseases focus in a particular area — the joints, for example, with rheumatoid arthritis. However, according to the Lupus Foundation of America Inc., lupus is a chronic inflammatory disease that can affect various parts of the body, especially the skin, joints, blood and kidneys.

"Lupus is not always predictable," says Dorothy K. Bloniarz, M.D., a rheumatologist with Crystal Run Healthcare. "It's an individualized disease."

"The disease is as unique as your own immune system," agrees Barbara Boyts, president of the Alliance for Lupus Research.

"Your body is fighting itself," says Karen.

"There is a portion of the cell membrane that the immune system recognizes as foreign so it attacks it — and the body can't turn it off," explains Bloniarz. "Lupus can affect multiple organ systems because it attacks connective tissue: skin, joints, blood vessels, heart and lung tissue, sometimes also the central nervous system."

And therein lies the main problem in diagnosis and treatment — lupus wears many masks.

The first symptom Karen noticed, when she was in her early 20s, was hair falling out after the birth of her children, now 23 and 20. The doctors told her it was from the pregnancy hormones.

Esposito remembers when she and Karen first started working together at Frontier Insurance Co. in Rock Hill some 12 years ago, Karen would occasionally complain of fatigue — a symptom often pooh-poohed by all those Type-A personalities who populate almost every workplace.

But the foot pain could not be brushed off. Surgery was performed to correct what was believed to be a nerve-related condition — and Karen's collective symptoms were now starting to garner some attention. It was a butterfly-shaped rash on her face that first led a dermatologist to suspect lupus.

Karen's lupus, however, was diagnosed only about nine years ago — years after her children were born. But when all her medical history is reviewed in the context of this diagnosis, it is believed that an eye complaint she had when she was just 11 or 12 was, in fact, the work of lupus.

Lupus commonly affects women of child-bearing age, says Bloniarz. "There is a clear genetic predisposition, but no one knows why the immune system turns on the body. Because it primarily affects women between the ages of 15 and 44, a hormonal influence is suspected. "» Right now, there is no cure."

"Lupus is nondiscriminatory," says Boyts. "Although 90 percent of those with lupus are women, and it is three times more common in African-American women, invariably whenever I'm speaking, I'll be approached by a young man who says he has lupus."

While it appears there are more people being diagnosed with lupus, Boyts says they're not certain if there actually is a higher rate of occurrence or just more people being definitively diagnosed.

"Karen is very open about it," says Esposito. "And since she's been diagnosed, we've learned of five or six people at work who have family members with lupus."

As with other chronic illnesses, most patients can coexist with this disease. The majority of patients do fairly well, says Bloniarz, who has treated more than 100 people with lupus.

"It's not unlike asthma, which also has an autoimmune component," she says. "Many people might only need their inhaler once every six months, while others die at a young age despite treatment.

"People with lupus need to be monitored very closely, and take very strong — although not overwhelmingly good — medications," says Bloniarz. "Steroids are the mainstay of flare-ups. Plaquenil (or hydroxychloroquine sulfate) is typically prescribed to control symptoms. The aggressiveness of the disease warrants this."

Bloniarz also stresses the importance of a healthy lifestyle — good nutrition and exercise — for her lupus patients, as well as avoiding the sun.

Although precise triggers have not been identified, along with sunlight, there is strong indication that hormones play a role, as well as links to certain medications, such as sulfur drugs.

Therefore, Bloniarz urges people with a family history of lupus to discuss thoroughly any and all medications that they might be taking or contemplating taking.

And because the cause is still a mystery, there is no way to prevent lupus — or any other autoimmune disease, for that matter, says Bloniarz.

The genetic link, though, concerns Karen. She worries about her daughter and granddaughter.

"She doesn't know what the future holds," says Esposito. "But her granddaughter keeps her going. She has a great spirit and strength — and the support of a wonderful family."

Karen is acutely aware of how her illness has altered the family dynamic. She cannot watch her young granddaughter alone, and the term "caregiver" accompanies "son," "daughter" and "husband."

"My husband has been amazing through this," says Karen.

"He's a saint," echoes Esposito, whose husband is also friends with Karen's husband — and their children are close, too.

Karen, blessed with a large extended family, continues to plan family events and activities — many of which get canceled. But she plans anyway.

"She's such a special person," says Esposito. "She remembers birthdays. "» When she can get out shopping, she loves to pick up meaningful gifts ... And she prefers to talk about other stuff on the phone, not herself. She cares about how you're doing. She's a really good friend, wife and mother."

Karen holds out hope that while today is tough, tomorrow she might be walking with a cane again, beating the disease into submission until research finds a cure.